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Article Dans Une Revue BONE Année : 2021

Low bone mass in Noonan syndrome children correlates with decreased muscle mass and low IGF-1 levels

Résumé

Although musculoskeletal abnormalities have long been described in patients with Noonan syndrome (NS), only a few studies have investigated the bone status of these patients. The aim of this retrospective observational study was to describe the bone health of children with NS. Thirty-five patients with a genetically confirmed diagnosis of NS were enrolled. We analyzed the axial skeleton (lumbar spine) using dual energy X-ray absorptiometry and the appendicular skeleton (hand) with the BoneXpert system. Bone metabolism markers, including mineral homeostasis parameters, serum 25-hydroxy vitamin D (25-OHD) levels and markers of bone formation and resorption were also reported. Compared to the general population, axial and appendicular bone mass was significantly decreased in children with NS (p < 0.0001). Serum 25-OHD levels were low in about half of the patients and were negatively correlated with age (r = -0.52; p < 0.0001). Patients with NS exhibited reduced bone formation marker levels and increased bone resorption marker levels (p < 0.0001). No gender difference or genotype-phenotype correlations were found for the different bone parameters. Muscle mass and, to a lesser extent, serum insulin-like growth factor 1 (IGF-1) levels were independent predictors of whole-body bone mineral content (p < 0.0001 for both parameters; adjusted R2 = 0.97). In conclusion, bone mass is reduced in children with NS and correlates with decreased muscle mass and low serum IGF-1 levels. These data justify addressing all potential threats to bone health including sufficient calcium and vitamin D intake, regular physical exercise, and hormone replacement therapy.
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hal-03781177 , version 1 (16-10-2023)

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Marine Delagrange, Vanessa Rousseau, Catie Cessans, Catherine Pienkowski, Isabelle Oliver, et al.. Low bone mass in Noonan syndrome children correlates with decreased muscle mass and low IGF-1 levels. BONE, 2021, 153, pp.116170. ⟨10.1016/j.bone.2021.116170⟩. ⟨hal-03781177⟩
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